A 9-year-old spayed female Golden Retriever was examined because of progressive hind limb lameness. Magnetic resonance imaging of the thoracic and lumbar portions of the vertebral column revealed a focal, contrast-enhancing, intramedullary spinal cord mass. The history, signalment, and magnetic resonance findings were suggestive of spinal cord neoplasia. A hemilaminectomy, durotomy, and longitudinal myelotomy were performed, and a 1 X 1-cm mass that contained numerous blood vessels was removed with blunt dissection. Results of histologic examination and immunohistochemical staining of the mass suggested that it was a hamartoma. The dog improved after surgery, with no evidence of a recurrence of clinical signs 14 months after surgery. Vascular malformations of the CNS in dogs include hamartomas, hemangiomas, angiomas, hemangioblastomas, meningocerebral hemangiomatosis, and arteriovenous malformations. A hamartoma is a non-neoplastic overgrowth of cells or an improper proportion of cells that are normally in the involved tissue. Although magnetic resonance imaging may be helpful in determining the extent of the lesion in dogs with vascular malforrmations, it cannot be used to distinguish neoplastic from non-neoplastic formations. Excision may result in a good outcome for dogs with an intramedullary spinal cord hamartoma.